New Liver Provides a Second Chance for Journey

Brittany’s daughter, Journey, entered the world without complications—as a healthy and happy 5-pound, 5-ounce baby. But that didn’t stop the new mom’s alarm bells from going off almost instantly.

“She was little when she was born, just a hair away from being a preemie,” Brittany said. “Yet I was induced at 41 weeks. So why had she stopped gaining weight?”

Shortly after birth, Journey developed jaundice, which is a yellowing of the skin and eyes due to a high level of bilirubin—a pigment produced by the breakdown of red blood cells—in the bloodstream.

After a few days, Brittany and her husband, LaAnder, were cleared to take their daughter home, but Brittany’s “mommy instinct” wouldn’t quit.

“I realized she wasn’t gaining weight as she should, but her belly was getting bigger,” she said, noting that Journey’s eyes also started to yellow when she was about 6 weeks old. “I told the doctor she looked like a frog to me. She had a big belly but thin legs and arms.”

Still, Journey’s pediatrician said that her liver might be taking longer to adjust to breast milk and to give it time.

Journey’s eyes didn’t get better, though. They got darker. So, when Journey was 2 months old, Brittany took her back to the doctor, who ordered a blood test.

“They did the labs over the weekend,” Brittany recalled. “That Monday, they called and said you need to go to the emergency department immediately.”

Confronting a Complex Condition

Journey’s diagnosis was biliary atresia, a rare disease affecting a baby’s liver and bile ducts. The condition blocks bile flow from the liver to the intestine, causing it to become trapped inside the liver. Without treatment, biliary atresia can lead to liver damage and scarring—and, eventually, liver failure.

The doctor told Brittany and LaAnder that their daughter needed an emergency surgery called a Kasai procedure, which is designed to create a pathway for bile from the liver to the intestine. While the Kasai procedure is successful for most patients, it wasn’t successful for Journey.

“It worsened things tremendously,” Brittany said.

Journey’s abdomen filled with fluid, causing her belly to become even more distended. A stitch securing the intestine had popped, and the fluid had started leaking out of her biopsy site—as well as from her belly button and from the Kasai’s primary incision site—causing an infection. 

“It was very traumatic,” Brittany said. “At that point, I knew we were going to be heading for a transplant before she was even added to the list.”

Finding a Leader in Liver Transplant

Biliary atresia is the most common reason children in the United States need a liver transplant. Brittany began researching, quickly realizing that Cincinnati Children’s was a leader in liver transplantation.

“I realized that the hospital where Journey was being treated performed about six liver transplants a year— compared to Cincinnati Children’s, which performed about 36,” Brittany said. “So, I knew that for Journey to have a shot, it would need to be in Cincinnati.”

Brittany and her family lived in Phoenix, but she and LaAnder had lived in Columbus, Ohio, previously.  LaAnder was from there, so they were familiar with Cincinnati Children’s, located less than 2 hours away.

Once Journey was cleared for transfer, Brittany and her daughter headed to Cincinnati. 

A Turning Point and Two Transplants

“Things were different immediately at Cincinnati Children’s,” Brittany said. “There was a sense of urgency.”

Alexander Miethke, MD, is the medical director of Cincinnati Children’s Liver Transplant Program and Journey’s primary liver doctor. He told Brittany that Journey would need a transplant within four weeks, confirming what Brittany knew to be true: “My daughter was the sickest of the sick.”

But Dr. Miethke also gave Brittany something to smile about. “He told me that Journey could have 7 milliliters an hour of breast milk,” she said. 

At the time, Journey used a gastric feeding tube to receive medicine directly to her stomach, but it didn’t deliver food. Due to the fluid in her abdomen and constant vomiting, Journey hadn’t had any food in her stomach for weeks.

To improve Journey's quality of life before transplant, Dr. Miethke decided to place Journey's tube further into her small intestine, avoiding the stomach. This was never presented as an option at the other hospital, Brittany said, and proved to be a crucial turning point for Journey. 

"That's what made the encounter so special. Instead of only getting nutrition through an IV, she was also getting some real food into her gut, something she had been missing," Brittany said.

“It was amazing, just getting that tiny bit of nutrition, that tiny bit of breast milk through her feeding tube, made Journey perk up and start to come back to life. She started to giggle a little bit. She was able to have 'real' nutrition again. She improved while waiting for the transplant. And it gave me some joy as a mom.”

Soon, Brittany learned that there was a liver ready for Journey. The surgery was scheduled, and LaAnder flew to Cincinnati to be there. Unfortunately, the transplant failed.

“Having a transplant isn’t necessarily a golden parachute or a guaranteed win,” said Anna Peters, MD, PhD, one of Journey’s doctors who specializes in pediatric chronic liver disease and liver transplantation. “It’s relatively rare for an organ that appears healthy to not work once it’s transplanted, but it’s still not an unexpected complication.”

Journey’s kidneys also failed, and Brittany and LaAnder began preparing for the worst possible scenario. Thankfully, just 13 days later, Journey got another chance with a second liver transplant. Although she went into cardiac arrest on her way into the operating room, Journey was saved by her team of surgeons and nurses. 

And this time, the transplant took. 

“The liver isn’t something that’s easy to replace, but with the help of nephrology and continuous renal therapy, and many different divisions coordinating their efforts, we were able to give Journey—a patient with multi-organ failure—another chance,” Dr. Miethke said. 

Strength and Resilience

Although Journey suffered a few complications and setbacks, the second transplant was a true turning point for the family. “Things started to get better,” Brittany said.

She and LaAnder eventually decided to move their young family to Columbus, to be closer to both family and to Cincinnati Children’s. Now a typical 2-year-old, Journey “is curious about the world and testing her boundaries,” Brittany said.

Because biliary atresia is a chronic, lifelong condition, Journey will need to take medications for the rest of her life. “But brushing your teeth is lifelong,” Brittany said, “and taking medications is a night-and-day difference from tubes and drains.”

Journey loves to sing, dance, read and cuddle. She is Brittany’s and LaAnder’s “greatest joy.”

“Her parents show me videos of this bubbly, happy, interactive toddler who’s playing and walking and eating and doing all of these things that she might not have been able to do even if the initial transplant had gone well,” Dr. Peters said. “Journey’s recovery has truly been remarkable.”

Brittany gives much of the credit to Journey’s care team: “You bring your anxieties into this high-stakes situation, and they have a calmness and sureness about them that allows you to relax—because you know they can handle it,” she said, adding that she’ll also never forget Journey’s donors. “Without their selfless gift, Journey wouldn’t be here.”

Yet a huge part of Journey’s success, according to her doctors, is her parents’ involvement.

“They’re incredibly loving and dedicated to their child,” Dr. Peters said. “And they’re willing to pull out all the stops, no matter what, to give her the best life and the best quality of life.”

Dr. Miethke agrees: “This is primarily a story about the resilience of a family,” he said. “My role was merely matching the strengths of our organization to the strength and resilience of this incredible family.”

(Published July 2024)